Primary Sclerosing Cholangitis (PSC) is a chronic disorder of the liver causing jaundice and liver damage. When Cystic Fibrosis affects the liver, the damaged liver looks like the liver in PSC. This study is designed to answer the question whether isolated PSC may be a form of CF only in the liver

CF was until recently, thought to be a multi-organ disease. However, there are now recognized non-classical presentations of CF involving a few organs only and now there is well established data on single -organ involvement including Congenital Absence of the Vas Deferens and recurrent pancreatitis.

CF and PSC have several features in common. They both affect intrahepatic bile ducts by inspisated biliary secretions, chronic inflammation and fibrosis. Is PSC the "single-organ presentation" of CF? Patients with PSC will undergo testing of the CF protein channel by nasal potential testing. This test is performed by insering a thin plastic tube 2 to 3 cms into the nostril and chloride transport can be measured. This test may shows that the patient has an abnormality in the CF protein channel.

Eligibility

Ages Eligible for Study:

10 Years and older

Genders Eligible for Study:

Both

Criteria

Inclusion Criteria:

Age > 10 years

Patients diagnosed as Non-IBD PSC

Patients diagnosed as IBD PSC

Exclusion Criteria:

Age < 10 years

Contacts and Locations

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study.
To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below.
For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00476814